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Mechanisms In Duchenne Muscular Dystrophy Pathophysiology And Treatment
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Book Synopsis Mechanisms in Duchenne Muscular Dystrophy Pathophysiology and Treatment by : Atsushi Asakura
Download or read book Mechanisms in Duchenne Muscular Dystrophy Pathophysiology and Treatment written by Atsushi Asakura and published by Frontiers Media SA. This book was released on 2023-09-07 with total page 150 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis Molecular Mechanisms of Muscular Dystrophies by : Steve J. Winder
Download or read book Molecular Mechanisms of Muscular Dystrophies written by Steve J. Winder and published by CRC Press. This book was released on 2006-01-26 with total page 250 pages. Available in PDF, EPUB and Kindle. Book excerpt: There is no doubt that the study of the muscular dystrophies in recent years has been exciting and rewarding. It has attracted the attention of many investigators of international repute, and this is reflected in the various contributions to this volume. Molecular Mechanisms of Muscular Dystrophies represents a personal collection of chapters cover
Book Synopsis Mechanisms in Prednisone-treated Duchenne Muscular Dystrophy Patients by : Koreen Lynn Burrow
Download or read book Mechanisms in Prednisone-treated Duchenne Muscular Dystrophy Patients written by Koreen Lynn Burrow and published by . This book was released on 1992 with total page 114 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis Pathophysiological Mechanisms of Sarcopenia in Aging and in Muscular Dystrophy: A Translational Approach by : Luciano Merlini
Download or read book Pathophysiological Mechanisms of Sarcopenia in Aging and in Muscular Dystrophy: A Translational Approach written by Luciano Merlini and published by Frontiers Media SA. This book was released on 2016-01-18 with total page 250 pages. Available in PDF, EPUB and Kindle. Book excerpt: Loss of muscle mass and increased fibrosis characterize both sarcopenia of aging and muscular dystrophy. Research is increasingly showing that these two conditions also share several pathophysiological mechanisms, including mitochondrial dysfunction, increased apoptosis, abnormal modulation of autophagy, decline in satellite cells, increased generation of reactive oxygen species, and abnormal regulation of signaling and stress response pathways. This Research Topic will cover several mechanisms involved in aging and dystrophic sarcopenia and explore the therapeutic potential of various strategies for intervention.
Book Synopsis New Insights Into the Disease Mechanisms of Duchenne Muscular Dystrophy Through Analyses of the Dystrophin, I[kappa]B[beta], and CASK Proteins by : Katherine Lynn Gardner
Download or read book New Insights Into the Disease Mechanisms of Duchenne Muscular Dystrophy Through Analyses of the Dystrophin, I[kappa]B[beta], and CASK Proteins written by Katherine Lynn Gardner and published by . This book was released on 2006 with total page 163 pages. Available in PDF, EPUB and Kindle. Book excerpt: Abstract: Duchenne muscular dystrophy (DMD) is a severe muscle wasting disease caused by the lack of functional dystrophin. Despite research focused on the replacement of the defective gene and the structure and function of dystrophin, there is no effective treatment of cure for this inevitably fatal disease. Rescue of dystrophin deficient, mdx, and utrophin/dystrophin deficient, dko, mouse models by the reintroduction of dystrophin has validated gene therapy as a therapeutic approach for DMD. Unfortunately the gene exceeds the capacity of adeno-associated viral (AAV) vectors. We investigated the expression of required dystrophin domains from two molecules as a means of expanding AAV capacity. Although the dystrophin proteins co-localize at the membrane, no improvement of dystrophic pathology is observed. Therefore, trans introduction of overlapping, truncated dystrophin proteins cannot be used to overcome cloning capacity limitations. The signaling mechanisms that lead from dystrophin deficiency to clinical DMD are largely unknown. We also conducted a proteomic analysis of phosphorylation differences between mdx and dko skeletal muscle and an immunoblot based examination of known signaling pathways. A serine phosphoprotein was observed in dko mitochondrial and microsome fractions but absent from corresponding mdx preparations. Unfortunately protein identification was inhibited by the abundance of actin in muscle. The nuclear factor-[kappa]B inhibitor, I[kappa]B[beta], was observed to exhibit an increase presence in dko muscle hinged upon the additional presence of both a nuclear and cytosolic form of the protein. Finally, we examined the neuromuscular junction (NMJ) protein, Calmodulin-associated serine/threonine kinase (CASK) in the cellular components of the NMJ. CASK exhibits a developmentally regulated localization in the C2C12 myogenic cell line. It is observed in the nuclei of proliferating myoblasts, but is excluded to the cytosol in differentiating myotubes. CASK also exhibits a nuclear presence in a motor neuron-neuroblastoma hybrid cell line suggesting a role in nuclei of neurons in the peripheral nervous system. Finally, we demonstrate that CASK does not co-cluster with acetylcholine receptors (AChR) in response to agrin or laminin treatment, suggesting a novel mechanism of recruitment to the NMJ. Understanding the development of the NMJ in normal skeletal muscle may provide insight into DMD and other neuromuscular diseases.
Book Synopsis Targeting Transcriptional and Translational Mechanisms to Enhance Utrophin A Expression as a Therapy for Duchenne Muscular Dystrophy by : Pedro Miura
Download or read book Targeting Transcriptional and Translational Mechanisms to Enhance Utrophin A Expression as a Therapy for Duchenne Muscular Dystrophy written by Pedro Miura and published by . This book was released on 2010 with total page 580 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis Duchenne Muscular Dystrophy by : Alan E. H. Emery
Download or read book Duchenne Muscular Dystrophy written by Alan E. H. Emery and published by OUP Oxford. This book was released on 2015-02-19 with total page 300 pages. Available in PDF, EPUB and Kindle. Book excerpt: Duchenne Muscular Dystrophy, an inherited and progressive muscle wasting disease, is one of the most common single gene disorders found in the developed world. In this fourth edition of the classic monograph on the topic, Alan Emery and Francesco Muntoni are joined by Rosaline Quinlivan, Consultant in Neuromuscular Disorders, to provide a thorough update on all aspects of the disorder. Recent understanding of the nature of the genetic defect responsible for Duchenne Muscular Dystrophy and isolation of the protein dystrophin has led to the development of new theories for the disease's pathogenesis. This new edition incorporates these advances from the field of molecular biology, and describes the resultant opportunities for screening, prenatal diagnosis, genetic counselling and from recent pioneering work with anti-sense oligonucleotides, the possibility of effective RNA therapy. Although there is still no cure for the disorder, there have been significant developments concerning the gene basis, publication of standards of care guidelines, and improvements in management leading to significantly longer survival, particularly with cardio-pulmonary care. The authors also investigate other forms of pharmacological, cellular and gene therapies. Duchenne Muscular Dystrophy will be essential reading not only for scientists and clinicians, but will also appeal to therapists and other professionals involved in the care of patients with muscular dystrophy.
Book Synopsis Skeletal Muscle Damage and Repair by : Peter M. Tiidus
Download or read book Skeletal Muscle Damage and Repair written by Peter M. Tiidus and published by Human Kinetics. This book was released on 2008 with total page 360 pages. Available in PDF, EPUB and Kindle. Book excerpt: Attempts to cover a wide range of both basic research and applied clinical topics related to skeletal muscle damage and repair mechanisms and their application. This book examines muscle damage and repair mechanisms and issues in specific populations including older adults and special populations.
Book Synopsis Exploration of the Mitochondria as a Potential Therapeutic Target in Duchenne Muscular Dystrophy by : Meghan Clare Hughes
Download or read book Exploration of the Mitochondria as a Potential Therapeutic Target in Duchenne Muscular Dystrophy written by Meghan Clare Hughes and published by . This book was released on 2019 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disease resulting from a mutation in the X-linked gene encoding the protein dystrophin. DMD is characterized by profound muscle weakness as degenerating muscle is replaced by fat and connective tissue. Early loss of ambulation followed by premature death due to cardiac and/or respiratory failure characterize the most debilitating aspects of DMD, a disease for which there is currently no cure. Limited success has been reported when treating DMD with gene based therapies. Current standard of care involves glucocorticoids, which target a secondary cellular myopathy; inflammation. While this line of treatment has provided promising benefits, these drugs present a variety of negative side effects for patients. As such, extensive research has been focused on identifying both therapeutic targets and corresponding novel therapies for the treatment of the DMD myopathy. The focus of this dissertation was to first determine the degree and precise mechanism of mitochondrial dysfunction in DMD followed by the evaluation of SBT-20, a mitochondrial-targeted peptide, as a therapeutic candidate for the treatment and prevention of DMD pathophysiology. In order to address these questions, we first comprehensively evaluated mitochondrial bioenergetics across a spectrum of oxidative and glycolytic muscles in the D2.B10-DMDmdx/2J mouse (D2.mdx) in early and late stages of disease progression. We then tested the efficacy of SBT-20 in improving both DMD myopathy in respiratory and skeletal muscles, as well as the underlying mechanism of mitochondrial dysfunction in dystrophic muscle. Our findings reveal that the mitochondrial H2O2 emission is elevated during impaired oxidative phosphorylation in cardiac, respiratory, oxidative and glycolytic muscle in young (4-week) and aged (52-week) D2.mdx mice and furthermore, that there is a specific defect in mtCK functionality in oxidative muscle. SBT-20 was effective in improving mitochondrial bioenergetics following short (4 weeks) and long (12 weeks) term treatment. This was associated with improved pathophysiology following short-term treatment. Taken together, this thesis identifies impairments in mitochondrial bioenergetics as a contributing factor to the pathophysiology in dystrophic muscle and further highlights SBT-20 as a promising therapy for the improvement of myopathy in DMD.
Book Synopsis Disorders of Voluntary Muscle by : George Karpati
Download or read book Disorders of Voluntary Muscle written by George Karpati and published by Cambridge University Press. This book was released on 2001-07-12 with total page 800 pages. Available in PDF, EPUB and Kindle. Book excerpt: Rewritten and redesigned, this remains the one essential text on the diseases of skeletal muscle.
Book Synopsis Myotonic Dystrophy by : Masanori P. Takahashi
Download or read book Myotonic Dystrophy written by Masanori P. Takahashi and published by Springer. This book was released on 2018-12-29 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book provides an essential overview combining both clinical and fundamental research advances in myotonic dystrophy. The pathomechanism of myotonic dystrophy has long been unclear, but in the past decade, our understanding has shifted to a novel disease mechanism concept: “RNA disease”. Parallel to these advances in elucidating the pathophysiology, translational research is also progressing rapidly. The current challenge lies in assessing the effectiveness of treatment, and as such, there is a growing interest in observational studies of the disease’s various clinical symptoms. The book introduces readers to the molecular mechanisms within each organ and the resultant clinical features, which are presented together. In particular, it focuses on the central nervous system, since the pathology of the brain (central nervous system manifestation) has rarely been addressed systematically and will pose a persistent challenge, even if therapies have greatly advanced in the future. In addition, the book addresses the latest developments, such as research using patient-derived iPS cells and therapeutic research. Myotonic Dystrophy provides essential information for neurologists and researchers with an interest in muscle disease, including muscular dystrophy. Furthermore, since the disease involves various complications of the brain, heart, metabolism, etc., the book will be of great value to clinicians and researchers in the cardiovascular sciences, endocrinology, diabetes, dementia, and neuropsychology, as well as genetic specialists.
Book Synopsis Muscle Homeostasis and Regeneration by : Antonio Musarò
Download or read book Muscle Homeostasis and Regeneration written by Antonio Musarò and published by MDPI. This book was released on 2020-11-20 with total page 500 pages. Available in PDF, EPUB and Kindle. Book excerpt: The book is a collection of original research and review articles addressing the intriguing field of the cellular and molecular players involved in muscle homeostasis and regeneration. One of the most ambitious aspirations of modern medical science is the possibility of regenerating any damaged part of the body, including skeletal muscle. This desire has prompted clinicians and researchers to search for innovative technologies aimed at replacing organs and tissues that are compromised. In this context, the papers, collected in this book, addressing a specific aspects of muscle homeostasis and regeneration under physiopathologic conditions, will help us to better understand the underlying mechanisms of muscle healing and will help to design more appropriate therapeutic approaches to improve muscle regeneration and to counteract muscle diseases.
Author :National Organization for Rare Disorders Publisher :Lippincott Williams & Wilkins ISBN 13 :9780781730631 Total Pages :982 pages Book Rating :4.7/5 (36 download)
Book Synopsis NORD Guide to Rare Disorders by : National Organization for Rare Disorders
Download or read book NORD Guide to Rare Disorders written by National Organization for Rare Disorders and published by Lippincott Williams & Wilkins. This book was released on 2003 with total page 982 pages. Available in PDF, EPUB and Kindle. Book excerpt: NORD Guide to Rare Disorders is a comprehensive, practical, authoritative guide to the diagnosis and management of more than 800 rare diseases. The diseases are discussed in a uniform, easy-to-follow format--a brief description, signs and symptoms, etiology, related disorders, epidemiology, standard treatment, investigational treatment, resources, and references.The book includes a complete directory of orphan drugs, a full-color atlas of visual diagnostic signs, and a Master Resource List of support groups and helpful organizations. An index of symptoms and key words offers physicians valuable assistance in finding the information they need quickly.
Download or read book Cardiomyopathy written by Gustav Mattsson and published by . This book was released on 2021 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: Cardiomyopathies are diseases of the heart muscle with diverse etiologies ranging from myocarditis to gene mutations. They are classified according to morphology and function, and then further categorized based on whether they are familial or non-familial and based on specific etiologies. This book examines the various cardiomyopathies, including arrhythmogenic cardiomyopathy, hypertrophic cardiomyopathy, and dilated cardiomyopathy, as well as their genetic basis.
Book Synopsis Investigations Into the Mechanisms of Degeneration in the Mdx Mouse, a Model for Duchenne Muscular Dystrophy by :
Download or read book Investigations Into the Mechanisms of Degeneration in the Mdx Mouse, a Model for Duchenne Muscular Dystrophy written by and published by . This book was released on 2006 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis Muscle Gene Therapy by : Dongsheng Duan
Download or read book Muscle Gene Therapy written by Dongsheng Duan and published by Springer Science & Business Media. This book was released on 2009-11-26 with total page 281 pages. Available in PDF, EPUB and Kindle. Book excerpt: Muscle disease represents an important health threat to the general population. There is essentially no cure. Gene therapy holds great promise to correct the genetic defects and eventually achieve full recovery in these diseases. Significant progresses have been made in the field of muscle gene therapy over the last few years. The development of novel gene delivery vectors has substantially enhanced specificity and efficiency of muscle gene delivery. The new knowledge on the immune response to viral vectors has added new insight in overcoming the immune obstacles. Most importantly, the field has finally moved from small experimental animal models to human patients. This book will bring together the leaders in the field of muscle gene transfer to provide an updated overview on the progress of muscle gene therapy. It will also highlight important clinical applications of muscle gene therapy.
Book Synopsis The Actin Cytoskeleton by : Brigitte M. Jockusch
Download or read book The Actin Cytoskeleton written by Brigitte M. Jockusch and published by Springer. This book was released on 2017-01-03 with total page 356 pages. Available in PDF, EPUB and Kindle. Book excerpt: Actin is one of the most abundant proteins and ubiquitously expressed in all eukaryotes. In recent years, the analysis of structure and function of such complexes has shed new light on actin's role in cellular and tissue morphogenesis, locomotion and various forms of intracellular motility, but also on its role in nuclear processes like chromatin architecture and transcription. Progress in understanding these different physiological phenomena, but also in unravelling the basis of actin-based pathophysiological processes has been made by combining video microscopy, molecular biology, genetics and biochemistry. Thus, the current research on actin, as ongoing in many international laboratories, is a "hot spot" in basic and translational research in life sciences. In this book on "The Actin Cytoskeleton", twelve internationally renowned authors present specific chapters that cover their recent work concerned with the various roles of actin mentioned above. This comprehensive volume is therefore an attractive handbook for teachers and students in many fields of medicine and pharmacology.
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